AUTHOR=Deslarzes Philip , Djafarrian Reza , Matter Maurice , La Rosa Stefano , Gengler Carole , Beck-Popovic Maja , Zingg Tobias 

TITLE=Neuroblastic Tumors of the Adrenal Gland in Elderly Patients: A Case Report and Review of the Literature

JOURNAL=Frontiers in Pediatrics

VOLUME=Volume 10 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/pediatrics/articles/10.3389/fped.2022.869518

DOI=10.3389/fped.2022.869518

ISSN=2296-2360

ABSTRACT=Abstract
Background
Neuroblastic neoplasms (NN) include ganglioneuromas (GN), 
ganglioneuroblastomas (GNB), and neuroblastomas (NB). They generally arise in 
childhood from primitive sympathetic ganglion cells. Their incidence in adults, 
especially among elderly, is extremely low. 

Case presentation
This is the case of a 74-year-old woman with history of abdominal pain, weakness 
and night sweating since several months. Blood pressure was normal. CT-scan 
showed a 10 cm left adrenal mass, without other pathologic findings. An open 
left-sided adrenalectomy was performed. Recovery was uneventful with hospital 
length of stay of 8 days.
Based on morphological, immunohistochemical, and molecular features the 
diagnosis was a nodular GNB. A positron emission tomography (PET) performed 
six weeks after the resection did not show any residual tumor or distant 
metastases. The patient was followed-up with annual clinical and radiological 
exams.

Conclusion 
This case presentation, associated with a review of the literature, illustrates the 
importance to include GNB NN in the preoperative differential diagnosis of 
adrenal tumors in adults and highlights the need for multidisciplinary patient
work-up and management.

Keywords
Neuroblastic tumor; adrenocortical tumor; ganglioneuroblastoma; case report; 
elderly patient; preoperative diagnosis; surgical and medical treatment; staging; 
; follow-up

List of abbreviations
Alpha-Thalassemia Mental Retardation-X (ATRX), Dehydroepiandrosterone 
(DHEA), Cluster of Differentiation - 99 (CD99), Fluorodeoxyglucose (FDG) 
Ganglioneuromas (GN), Ganglioneuroblastomas (GNB), General Practician (GP) 
Glial Fibrillary Acidic Protein (GFAP), Metaiodobenzylguanidine (MIBG), 
International Neuroblastoma Staging System (INSS), International 
Neuroblastoma Risk Group Staging System (INRGSS), Neuroblastic Neoplasms 
(NN), Neuroblastomas (NB), Microtubule Associated Protein 2 (MAP-2), 
3Myelocytomatosis oncogene (MYC), Overall Survival (OS), Positron Emission 
Tomography (PET), Splicing Factor 1 (SF1), Succinate Dehydrogenase (SDHB